Acute rheumatic fever (ARF) continues to cause a large burden of mortality and morbidity in low- and middle-income countries. It is less common in high-income countries, but continues to be seen in indigenous communities and during outbreaks.
Currently, no single test can diagnose ARF. Diagnosis is clinical and relies on the Jones criteria.
The 5 major manifestations of ARF are carditis, arthritis, chorea, erythema marginatum, and subcutaneous nodules, of which the most common are carditis and arthritis.
The Jones criteria were revised in 2015 to include separate criteria for low-risk and moderate- to high-risk populations and introduce the use of Doppler echocardiography to diagnose subclinical carditis.
While all other manifestations of ARF resolve without sequelae, carditis can lead to chronic rheumatic heart disease (RHD).
Secondary prophylaxis is thought to ameliorate the progression of ARF to chronic RHD, but even the mildest forms of carditis can result in chronic RHD and decreased survival.
The prognosis of established rheumatic valvular disease remains guarded, especially with severe disease.
Acute rheumatic fever (ARF) is an autoimmune disease that mostly occurs following group A streptococcal throat infection, although there is growing evidence of ARF following skin infection in certain regions of the world. It can affect multiple systems, including the joints, heart, brain, and skin. Only the effects on the heart can lead to permanent illness; chronic changes to the heart valves are referred to as chronic rheumatic heart disease (RHD). Without long-term penicillin secondary prophylaxis, ARF can recur, leading to cumulative damage to the cardiac valvular tissue.
History and exam
Key diagnostic factors
- joint pain
Other diagnostic factors
- recent sore throat or scarlet fever
- recent skin infection
- chest pain
- shortness of breath
- heart murmur
- pericardial rub
- signs of cardiac failure
- swollen joints
- emotional lability and personality changes
- jerky, uncoordinated choreiform movements
- inability to maintain protrusion of the tongue
- milkmaid's grip
- spooning sign
- pronator sign
- erythema marginatum
- subcutaneous nodules
- pregnancy or taking oral contraceptive pill
- overcrowded living quarters
- family history of rheumatic fever
- HLA association
- genetic susceptibility
- indigenous populations; Aboriginal Australian, Asian, and Pacific Islanders
- D8/17 B cell antigen positivity
1st investigations to order
- erythrocyte sedimentation rate (ESR)
- WBC count
- blood cultures
- chest x-ray
- throat culture
- rapid antigen test for group A streptococci
- anti-streptococcal serology
- rapid molecular test
monoarthritis in unconfirmed rheumatic fever
possible rheumatic fever
confirmed rheumatic fever
all patients following acute treatment
Liesl Zühlke, MBChB DCH FCPaeds Cert Card (Paeds) MPH FACC FESC MSc PhD
Vice-President South African Medical Research Council - Extramural Research and Internal Portfolio
Director Children's Heart Disease Research Unit
Paediatric Cardiologist, Division of Paediatric Cardiology, Department of Paediatrics
Red Cross Children's Hospital
Cape Heart Institute and Institute of Infectious Diseases and Molecular Medicine
Faculty of Health Sciences
University of Cape Town
LZ has been funded by the South African Medical Research Council, NRF, and through the African Research Leader award jointly by the UK Medical Research Council (MRC) and the UK Department for International Development (DFID) under the MRC/DFID Concordat agreement. LZ is a board member of the World Heart Federation, the NCD Alliance, and FoodForward South Africa. LZ an author of the UpToDate rheumatic heart disease topic. None of the above are competing interests.
John Lawrenson, null
Head of Clinical Unit
Paediatric Cardiology Service of the Western Cape
Red Cross Children's and Tygerberg Hospital
Stellenbosch University and University of Cape Town
JL declares that he has no competing interests.
Professor Liesl Zühlke and Professor John Lawrenson would like to gratefully acknowledge Dr Rachel Webb, Dr Andrew C. Steer, and Dr Jonathan Carapetis, previous contributors to this topic.
RW declares that she has no competing interests; she is an active researcher and clinician in acute rheumatic fever/rheumatic heart disease and is a co-investigator on a (non-industry) grant funded by the Health Research Council of New Zealand and gives educational talks and has prepared manuscripts on rheumatic fever solely in capacity as a University of Auckland academic and Paediatric Infectious Diseases Specialist. ACS and JC declare that they have no competing interests.
Salah Zaher, MD
Professor of Pediatrics
Division of Pediatric Cardiology
Faculty of Medicine
University of Alexandria
El Shatby Children's Hospital
SZ declares that she has no competing interests.
Nigel Wilson, FRACP
Paediatric Cardiologist/Interventional Cardiologist
Paediatric and Congenital Cardiac Services
Green Lane Clinical Services
Starship Children's Hospital
NW declares that he has no competing interests.
Andrea Summer, MD
Assistant Professor of Pediatrics
Medical University of South Carolina
AS declares that she has no competing interests.
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